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Expression of muscle cell surface antigen 5.1H11 in infantile or juvenile spinal muscular atrophy

Institute of Neurology, Queen Square, London, UK

Monoclonal antibody 5.1H11 reacts with a cell surface antigen of human myoblasts and myotubes in cell culture. We found that levels of this antigen decrease to background levels during development of fetal muscle, and this down-regulation occurs after innervation. Adult myofibers are uniformly negative for 5.1H11 reactivity. Denervated muscle fibers from cases of Werdnig-Hoffmann disease and Kugelberg-Welander syndrome expressed 5.1H 11 reactivity, while innervated fibers on the same sections were negative. Levels of 5.1H11 antigen therefore appear to be regulated by nerve in these spinal muscular atrophies.

Address correspondence and reprint requests to Dr. Walsh, Institute of Neurology, Queen Square, London WC1N 3BG, UK.

Supported by the Muscular Dystrophy Group of Great Britain and the Medical Research Council.

Accepted for publication December 18, 1985.

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